4-Trimethylammoniobutanoic acid

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4-Trimethylammoniobutanoic acid
2D structure for 4-Trimethylammoniobutanoic acid
Chemical Name	(4-hydroxy-4-oxobutyl)-trimethylazanium
Chemical Formula	C₇H₁₆NO₂
CAS Number	407-64-7
Chemical Information	HMDB01161
Biochemical Taxonomy	Carnitines
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Bile Acid Biosynthesis Butanoate Metabolism Fatty Acid Metabolism Glycerolipid Metabolism Glycolysis Lysine Degradation Propanoate Metabolism Pyruvate Metabolism Valine, Leucine and Isoleucine Degradation
Biofluid Location	Blood
Tissue Location	Not Available
Normal Biofluid Concentrations	Blood: 10.0 (0.5-20.0) umol/L
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Not Available
Other (Monogenic Disorders)	Carnitine deficiency, systemic primary OMIM: 212140
Abnormal Biofluid Concentrations	Not Available
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
4-Trimethylammoniobutanoic acid is a substrate for Gamma-butyrobetaine dioxygenase, Aldehyde dehydrogenase (dimeric NADP-preferring), Aldehyde dehydrogenase family 7 member A1, Aldehyde dehydrogenase 1A3, Aldehyde dehydrogenase (mitochondrial), Fatty aldehyde dehydrogenase, Aldehyde dehydrogenase X (mitochondrial) and 4-trimethylaminobutyraldehyde dehydrogenase.